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Excessive IL\u20101\u03b2 levels are detected in patients with systemic juvenile idiopathic arthritis (sJIA) and cytokine storm syndrome (CSS) with mutated and unmutated inflammasome components, raising questions on the mechanisms of IL\u20101\u03b2 regulation in these disorders.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      To investigate how the NLRP3 inflammasome is modulated in sJIA, we focused on Transmembrane protein 178 (Tmem178), a negative regulator of calcium levels in macrophages, and measured IL\u20101\u03b2 and caspase\u20101 activation in wild\u2010type (WT) and\n                      <jats:italic>Tmem178<\/jats:italic>\n                      <jats:sup>\u2212\/\u2212<\/jats:sup>\n                      macrophages after calcium chelators, silencing of Stim1, a component of store\u2010operated calcium entry (SOCE), or by expressing a Tmem178 mutant lacking the Stromal Interaction Molecule 1 (Stim1) binding site. Mitochondrial function in both genotypes was assessed by measuring oxidative respiration, mitochondrial reactive oxygen species (mtROS), and mitochondrial damage. CSS development was analyzed in\n                      <jats:italic>Perforin<\/jats:italic>\n                      <jats:sup>\u2212\/\u2212<\/jats:sup>\n                      \/\n                      <jats:italic>Tmem178<\/jats:italic>\n                      <jats:sup>\u2212\/\u2212<\/jats:sup>\n                      mice infected with lymphocytic choriomeningitis virus (LCMV) in which inflammasome or IL\u20101\u03b2 signaling was pharmacologically inhibited. Human\n                      <jats:italic>TMEM178<\/jats:italic>\n                      and\n                      <jats:italic>IL1B<\/jats:italic>\n                      transcripts were analyzed in data sets of whole blood and peripheral blood monocytes from healthy controls and patients with active sJIA.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      <jats:italic>TMEM178<\/jats:italic>\n                      levels are reduced in whole blood and monocytes from patients with sJIA while\n                      <jats:italic>IL1B<\/jats:italic>\n                      levels are increased. Accordingly,\n                      <jats:italic>Tmem178<\/jats:italic>\n                      <jats:sup>\u2212\/\u2212<\/jats:sup>\n                      macrophages produce elevated IL\u20101\u03b2 compared with WT cells. The elevated intracellular calcium levels after SOCE activation in\n                      <jats:italic>Tmem178<\/jats:italic>\n                      <jats:sup>\u2212\/\u2212<\/jats:sup>\n                      macrophages induce mitochondrial damage, release mtROS, and ultimately promote NLRP3 inflammasome activation. In vivo, inhibition of inflammasome or IL\u20101\u03b2 neutralization prolongs\n                      <jats:italic>Tmem178<\/jats:italic>\n                      <jats:sup>\u2212\/\u2212<\/jats:sup>\n                      mouse survival in LCMV\u2010induced CSS.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>\n                      Down\u2010regulation of\n                      <jats:italic>TMEM178<\/jats:italic>\n                      levels may represent a marker of disease activity and help identify patients who could benefit from inflammasome targeting.\n                    <\/jats:p>\n                    <jats:p>\n                      <jats:boxed-text content-type=\"graphic\" position=\"anchor\">\n                        <jats:graphic xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" mimetype=\"image\/png\" position=\"anchor\" specific-use=\"enlarged-web-image\" xlink:href=\"graphic\/art42666-toc-0001-m.png\">\n                          <jats:alt-text>image<\/jats:alt-text>\n                        <\/jats:graphic>\n                      <\/jats:boxed-text>\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/art.42666","type":"journal-article","created":{"date-parts":[[2023,8,3]],"date-time":"2023-08-03T05:34:58Z","timestamp":1691040898000},"page":"107-118","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Tmem178 Negatively Regulates\n                    <scp>IL<\/scp>\n                    \u20101\u03b2 Production Through Inhibition of the\n                    <scp>NLRP3<\/scp>\n                    Inflammasome"],"prefix":"10.1002","volume":"76","author":[{"given":"Kunjan","family":"Khanna","sequence":"first","affiliation":[{"name":"Washington University in St. Louis  St. Louis 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